在青少年局部性硬化症中,皮肤外表现的负担:文献综述
Ilaria Liguoro1, Gabriele Simonini2, Giorgia Martini3
1Pediatric Division, University Hospital S Maria della Misericordia, Udine, Italy.
Autoimmunity reviews
|April 6, 2025
概括
青少年局部性硬化症 (JLS) 影响皮肤纤维化儿童,在超过25%的病例中可能导致皮肤外表现 (ECM). 肌肉骨,神经和牙科问题是最常见的,影响生活质量.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 自免疫性疾病 自免疫性疾病
- 皮肤病学 皮肤病学
背景情况:
- 青少年局部性硬化症 (JLS) 是一种小儿自身免疫性疾病,导致皮肤和皮下组织纤维化.
- 外皮表现 (ECM) 影响约20%的JLS患者,导致严重的残疾和降低生活质量 (QoL).
研究的目的:
- 系统地审查现有的关于儿童诊断为青少年局部性硬化皮肤病 (JLS) 的儿科患者皮肤外表现 (ECM) 的文献.
- 分析研究,重点关注ECM对生活质量 (QoL) 的影响以及多学科方法在管理JLS患者中的作用.
主要方法:
- 在PubMed,Cochrane和Scopus数据库中进行了系统的文献搜索.
- 确定并选择了评估JLS儿童ECM的研究.
- 专门讨论QoL和多学科管理的论文被单独分析.
主要成果:
- 包括15篇涉及3604名儿童的论文,报告ECM在26.5%的病例中.
- 最常见的ECM是肌肉骨 (24%),神经 (10.3%) 和牙牙科 (7.6%).
- 六篇论文分析了QoL,三篇研究研究了JLS患者ECM的多学科团队管理.
结论:
- 近三分之一的JLS患者经历了皮肤病变以外的ECM,需要多学科护理.
- 支持多专业管理有效性的证据有限.
- ECM对质量生活的影响可能被低估;需要改进的评估工具.
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